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Cardiopulmonary Syndromes (PDQ®): Supportive care - Health Professional Information [NCI] - Superior Vena Cava Syndrome


SVCS is rare in children and appears at presentation in 12% of pediatric patients with malignant mediastinal tumors.[28,29] The etiology, diagnosis, and treatment of SVCS in children differs from that in adults. Whereas the most frequent cause of SVCS in adults is bronchogenic carcinoma,[3] in children the most frequent malignant cause of the syndrome is non-Hodgkin lymphoma. As in adults, a frequent nonmalignant cause is thrombosis from catheterization for venous access.[26]

A physical examination, chest radiograph, and the medical history of the patient are usually sufficient to establish a diagnosis of SVCS. If lymphoma or other malignant disease is suspected, it is desirable to obtain a tissue sample for diagnosis. However, the procedure to obtain the specimen may involve significant risk and may not be clinically feasible. Children with SVCS have a poor tolerance for the necessary general anesthesia because the accompanying cardiovascular and pulmonary changes aggravate the SVCS, often making intubation impossible. Also, extubation may be difficult or impossible, thus requiring prolonged airway provision (intubation). A CT scan of the chest to determine tracheal size, upright and supine echocardiography, and a flow volume loop may help evaluate anesthetic risk. Because anesthesia use is a serious risk, the diagnosis should be made with the least invasive means possible.[30] Published reports suggest a stepwise approach to diagnosis.[26]

When a malignant mass is the cause of the SVCS, the situation may be a medical emergency with no opportunity to establish a tissue diagnosis. In these cases, the most appropriate course may be to initiate empiric therapy prior to biopsy. The traditional empiric therapy is irradiation, with the daily dose governed by the presumed radiosensitivity of the tumor. After irradiation, respiratory deterioration from the apparent tracheal swelling may occur because of the inability of narrow lumens in children to accommodate edema and because of the greater degree of edema at onset, which is due to the rapid speed at which tumors grow in children. In these situations, a course of prednisone at 10 mg/m2 of body surface area 4 times per day may be necessary.[26]

In addition to radiation, empiric therapy of SVCS has included chemotherapeutic agents incorporating steroids, cyclophosphamide, or both in combination with an anthracycline and vincristine.[26] If the tumor fails to respond, it may be a benign lesion.

If surgery becomes necessary, it should be performed with the patient in the semi-Fowler's position, allowing the surgeon the ability to rapidly change the patient's position to lateral or prone. Cardiopulmonary bypass facilities and a rigid bronchoscope should be available in a standby capacity.[30]


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  16. Baker GL, Barnes HJ: Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care 1 (1): 54-64, 1992.
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  18. Würschmidt F, Bünemann H, Heilmann HP: Small cell lung cancer with and without superior vena cava syndrome: a multivariate analysis of prognostic factors in 408 cases. Int J Radiat Oncol Biol Phys 33 (1): 77-82, 1995.
  19. Rodrigues CI, Njo KH, Karim AB: Hypofractionated radiation therapy in the treatment of superior vena cava syndrome. Lung Cancer 10 (3-4): 221-8, 1993.
  20. Tanigawa N, Sawada S, Mishima K, et al.: Clinical outcome of stenting in superior vena cava syndrome associated with malignant tumors. Comparison with conventional treatment. Acta Radiol 39 (6): 669-74, 1998.
  21. Nicholson AA, Ettles DF, Arnold A, et al.: Treatment of malignant superior vena cava obstruction: metal stents or radiation therapy. J Vasc Interv Radiol 8 (5): 781-8, 1997 Sep-Oct.
  22. Dyet JF, Nicholson AA, Cook AM: The use of the Wallstent endovascular prosthesis in the treatment of malignant obstruction of the superior vena cava. Clin Radiol 48 (6): 381-5, 1993.
  23. Irving JD, Dondelinger RF, Reidy JF, et al.: Gianturco self-expanding stents: clinical experience in the vena cava and large veins. Cardiovasc Intervent Radiol 15 (5): 328-33, 1992 Sep-Oct.
  24. Doty DB: Bypass of superior vena cava: Six years' experience with spiral vein graft for obstruction of superior vena cava due to benign and malignant disease. J Thorac Cardiovasc Surg 83 (3): 326-38, 1982.
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  26. Lange B, O'Neill JA, D'Angio G, et al.: Oncologic emergencies. In: Pizzo PA, Poplack DG: Principles and Practice of Pediatric Oncology. 2nd ed. Philadelphia, Pa: JB Lippincott, 1993, pp 951-972.
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WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
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